Title:The Patient with a Single Cardiac Ventricle
Volume: 8
Issue: 3
Author(s): Christopher W. Mastropietro, Jeff A. Clark, Lisa M. Grimaldi, James S. Killinger and Marc Richmond
Affiliation:
Keywords:
Congenital heart disease, hypoplastic left heart syndrome, Fontan procedure, Norwood procedure, single ventricle,
superior cavopulmonary anastomosis
Abstract: Patients born with a single cardiac ventricle are one of the most complex and challenging subgroups of congenital
heart disease to manage, from their initial diagnosis to their long-term post-surgical sequelae. Advances in antenatal
detection, operative techniques, and post-operative strategies have led to improved outcomes over the past two decades,
yet morbidity and mortality remain high relative to other congenital heart lesions. Optimal management and outcome
depend in part on a thorough understanding of the anatomy and physiology unique to these infants by all caregivers
that may be involved including neonatologists, primary care pediatricians, emergency medicine physicians, and pediatric
intensivists. This review will discuss in detail the course of these infants, from their birth through to their three stage surgical
palliations and beyond. This review will also highlight many of the most recent medical and surgical innovations
available to these infants.